Darya's disease

Darier's disease( psorospermoz, dyskeratosis follicularis, keratosis vegetating) - Genetic chronic skin disease characterized by the appearance of conical brownish or yellowish-brown nodules specific diameter up to five millimeters. These nodules in its main part associated with hair follicles and their surface is covered with thick crusts grayish yellow

Symptoms

- Having multiple dense skin papules( nodules) mostly spherical shape with a diameter of two to five millimeters, with scaly rough surface. The color of these formations varies from the color of normal skin to grayish brown. Often papules merge, resulting in formation of oozing lesions,
skin located in the folds - to the nail plate appears white and red longitudinal stripes, in some cases, there is their longitudinal splitting
- may damage the larynx mucous membranes, throat, mouth, esophagus, directGuts, vulva and vagina
- Subungual hyperkeratosis may develop

Causes of

The main cause of this disease is considered to be a defect in both sexesth gene( fifty per cent of cases transmitted to children by hereditary line).In healthy children, a sick parent is born exceptionally healthy offspring. Darya's disease is transmitted by the type of vertical inheritance( if the reproductive function is not violated).

The main risk factor is genetic predisposition

main forms of Darier disease

- Localized form: asymmetrically located on localized areas elongated lesions
- Classic follicular dyskeratosis: arranged symmetrically with nodular lesions on the scalp, face, behind the ears, between the shoulder blades, in the field ofSternum and in large folds
- Isolated form: in addition to typical nodules on the back of the feet and brushes appear warty type polyhedronPapules that form plaques when merged
- Vesicular coloform: in addition to papules, bubbles are filled with transparent contents. Such foci occur on the lateral surfaces of the neck and in large folds. On the soles and palms may develop a point keratosis( the appearance of horny areas)

In most cases diagnosed solely on clinical grounds, but in case of doubt as to the correctness of the established diagnosis is performed histological examination of affected skin

Treatment

- Supplementation of vitamin A
- ReceptionKeratolytic drugs
- In order to reduce the risk of side effects and increase the effectiveness of vitamin A therapy, hepatoprotectomoat and vitamins
- Laser
- Cryotherapy
- Diathermocoagulation
- Physiotherapy( radon and sulphide bath)
Treatment of this disease is usually limited to ambulatory, and only in cases of acute patients are sent to hospital dermatological.

It is also necessary to know that in case of untimely treatment of Darje's disease serious complications can develop:
- Mental retardation
- Viral or bacterial skin infection
- Epilepsy
- Various skeletal anomalies
- Endocrinopathy
- Eye damage
The prognosis for life is quite favorable, andTo avoid possible exacerbations, it is necessary to avoid UV irradiation and prolonged sun exposure, if possible.